Advances in the management of congenital and infantile cataract.

Congenital and infantile cataracts produce deprivation amblyopia and can thus cause lifelong visual impairment. Successful management is dependent on early diagnosis and referral for surgery when indicated. Accurate optical rehabilitation and postoperative supervision are essential.The timing of surgery and its relationship to the duration of deprivation is important. Unilateral congenital cataract surgery within 6 weeks of birth produces the best outcomes. The equivalent 'latent' period for bilateral visual deprivation may be longer at around 10 weeks. Visual deprivation has a significant impact on the development of fixation stability. Major form deprivation, even after early surgery, leads to nystagmus. This is mostly manifest latent nystagmus (MLN). The latent period for fixation stability may be as short as 3 weeks. Preoperative congenital nystagmus (CN) can convert to more benign MLN after surgery. Infantile IOL implantation is becoming increasingly accepted. A satisfactory long-term refractive result requires that allowance be made for childhood axial growth and myopic shift. In a series of 25 infants (33 eyes) implanted before 12 months of age, the mean myopic shift at 12 months was 4.83 D. This increased to 5.3 D in infants implanted before 10 weeks. The initial desired refractive outcome following IOL implantation is thus hypermetropia, with the degree dependent on the age of the child. Glaucoma or ocular hypertension is a common complication following paediatric cataract surgery. Microphthalmia and surgery in early infancy are risk factors. Tonometry results may be influenced by the increased corneal thickness seen in aphakic and pseudophakic children. The long-term prognosis of eyes with aphakic glaucoma is not necessarily poor but intraocular pressure control may require three or more medications. Surgical intervention appears to be necessary in over a quarter of eyes. Posterior capsule opacification (PCO) is common in infants undergoing primary lens implantation. Primary capsulotomy and anterior vitrectomy reduce the risk of PCO. In the absence of anterior vitrectomy, primary posterior capsulotomy does not prevent visual axis opacification. Further developments will continue to be driven by clinical research. The prevention of capsule opacification and cellular proliferation may in future be achieved by the use of devices to specifically target epithelial cells at surgery.

Modulation of saccadic intrusions by exogenous and endogenous attention.

Primary gaze fixation in healthy individuals is frequently interrupted by microsaccades and saccadic intrusions (SI). The neural systems responsible for the control of attention and eye movements are believed to overlap and in line with this, the behaviour of microsaccades appears to be affected by exogenous and endogenous attention shifts. In the current work we wished to establish whether SI would also be influenced by attention in order to provide evidence that SI and microsaccades exhibit similar behaviour and further investigate the extent of overlap between attention and eye movement systems. Twelve participants performed a cue-target task where they were cued exogenously or endogenously and had to respond to the appearance of a peripheral target with either a button press or saccade. Our results replicate earlier microsaccade research, indicating that SI are also influenced by exogenous and endogenous attention. In all conditions, SI frequency initially decreased following the cue, then rose to a maximum before falling to below baseline levels. Following the exogenous cue, SI were more frequently directed away from the cue as predicted by inhibition of return. Additionally, SI direction following the endogenous cue was biased towards the cue for the saccadic response mode only, suggesting that the degree to which the eye movement and attention systems overlap depends on whether an eye movement is required. In summary, our findings indicate that SI characteristics are modulated by exogenous and endogenous attention and in a similar way to microsaccades, suggesting that SI and microsaccades may lie on a continuum of fixational instabilities. Furthermore, as with microsaccades, SI are likely to provide additional insights into the relationship between attention and the oculomotor systems.

Grading infantile cataracts.

PURPOSE: To introduce and describe two methods of grading the severity of infantile cataracts, and thereby propose a useful clinical guide for early surgical intervention. METHODS: Thirty-three subjects, aged 1 week to 8 years, participated in the study. Twenty-two were evaluated soon after birth (1 week), and 11 in childhood (3-8 years). All had isolated infantile cataracts, of which 16 were bilateral and 17 unilateral. Nine cataract types were examined; nuclear (n = 9), lamellar (n = 9), posterior lenticonus (n = 4), persistent hyperplastic primary vitreous (n = 4), posterior polar (n = 3) and single cases of total, cortical, sutural and anterior polar. Grading the infantile cataracts was performed subjectively based on the cataract morphology, density and position using an 11-point (0-10) ordinal scale. Objective measures of the cataracts were performed by scanning and then digitising photo-slit lamp images to provide cataract intensity profiles. Subjects without cataracts acted as controls. RESULTS: Subjective gradings of 0 and 10 were assigned to the clear, cataract-free lens and the total cataract, respectively. Fixed grades of 1 (anterior polar, sutural) and 6 (posterior polar) were assigned to the three remaining cataracts with static morphologies. The five cataracts which were all progressive were given grading ranges, reflecting the initial and likely final morphological states. Objective measures were found to be valuable in indicating the exact position and relative density of the cataract, as well as accurately defining boundaries. CONCLUSIONS: The magnitude and severity of infantile cataracts can be usefully characterised by an 11-point ordinal subjective grading scale. Although subjective grading alone is satisfactory, it can be greatly assisted by objective measures, particularly in the documentation of cataract progression. Cataracts assigned grades 1-4 were considered minor obstructions to vision and therefore not candidates for early surgery. Cataracts graded 5 and above were considered major visual defects, and ideally should be removed early in life.

Nonlinear time series analysis of jerk congenital nystagmus.

Nonlinear dynamics provides a complementary framework to control theory for the quantitative analysis of the oculomotor control system. This paper presents a number of findings relating to the aetiology and mechanics of the pathological ocular oscillation jerk congenital nystagmus (jerk CN). A range of time series analysis techniques were applied to recorded jerk CN waveforms, and also to simulated jerk waveforms produced by an established model in which the oscillations are a consequence of an unstable neural integrator. The results of the analysis were then interpreted within the framework of a generalised model of the unforced oculomotor system.This work suggests that for jerk oscillations, the origin of the instability lies in one of the five oculomotor subsystems, rather than in the final common pathway (the neural integrator and muscle plant). Additionally, experimental estimates of the linearised foveation dynamics imply that a refixating fast phase induced by a near-homoclinic trajectory will result in periodic oscillations. Local dimension calculations show that the dimension of the experimental jerk CN data increases during the fast phase, indicating that the oscillations are not periodic, and hence that the refixation mechanism is of greater complexity than a homoclinic reinjection. The dimension increase is hypothesised to result either from a signal-dependent noise process in the saccadic system, or the activation of additional oculomotor components at the beginning of the fast phase. The modification of a recent saccadic system model to incorporate biologically realistic signal-dependent noise is suggested, in order to test the first of these hypotheses.

Paying attention to saccadic intrusions.

Fixation to a target in primary gaze is invariably interrupted by physiological conjugate saccadic intrusions (SI). These small idiosyncratic eye movements (usually <1 degrees in amplitude) take the form of an initial horizontal fast eye movement away from the desired eye position, followed after a variable duration by a return saccade or drift. As the aetiology of SI is still unclear, it was the aim of this study to investigate whether SI are related to exogenous or endogenous attentional processes. This was achieved by varying (a) the "bottom-up" target viewing conditions (target presence, servo control of the target, target background, target size) and (b) the 'top-down' attentional state (instruction change--'look' or 'hold eyes steady' and passive fixation versus active--'respond to change' fixation) in 13 subjects (the number of participants in each task varied between 7 and 11). We also manipulated the orientation of pure exogenous attention through a cue-target task, during which subjects were required to respond to a target, preceded by a non-informative cue by either pressing a button or making a saccade towards the target. SI amplitude, duration, frequency and direction were measured. SI amplitude was found to be significantly higher when the target was absent and SI frequency significantly lower during open loop conditions. Target size and background influenced SI behaviour in an idiosyncratic manner, although there was a trend for subjects to exhibit lower SI frequencies and amplitudes when a patterned background was present and larger SI amplitudes with larger target sizes. SI frequency decreased during the "hold eyes steady" passive command as well as during active fixation but SI direction was not influenced by the exogenous cue-target task. These results suggest that SI are related to endogenous rather than exogenous attention mechanisms. Our experiments lead us to propose that SI represent shifts in endogenous attention that reflect a baseline attention state present during laboratory fixation tasks and may prove to be a useful tool to explore higher cortical control of fixation.

Eye movement instabilities and nystagmus can be predicted by a nonlinear dynamics model of the saccadic system.

The study of eye movements and oculomotor disorders has, for four decades, greatly benefitted from the application of control theoretic concepts. This paper is an example of a complementary approach based on the theory of nonlinear dynamical systems. Recently, a nonlinear dynamics model of the saccadic system was developed, comprising a symmetric piecewise-smooth system of six first-order autonomous ordinary differential equations. A preliminary numerical investigation of the model revealed that in addition to generating normal saccades, it could also simulate inaccurate saccades, and the oscillatory instability known as congenital nystagmus (CN). By varying the parameters of the model, several types of CN oscillations were produced, including jerk, bidirectional jerk and pendular nystagmus. The aim of this study was to investigate the bifurcations and attractors of the model, in order to obtain a classification of the simulated oculomotor behaviours. The application of standard stability analysis techniques, together with numerical work, revealed that the equations have a rich bifurcation structure. In addition to Hopf, homoclinic and saddlenode bifurcations organised by a Takens-Bogdanov point, the equations can undergo nonsmooth pitchfork bifurcations and nonsmooth gluing bifurcations. Evidence was also found for the existence of Hopf-initiated canards. The simulated jerk CN waveforms were found to correspond to a pair of post-canard symmetry-related limit cycles, which exist in regions of parameter space where the equations are a slow-fast system. The slow and fast phases of the simulated oscillations were attributed to the geometry of the corresponding slow manifold. The simulated bidirectional jerk and pendular waveforms were attributed to a symmetry invariant limit cycle produced by the gluing of the asymmetric cycles. In contrast to control models of the oculomotor system, the bifurcation analysis places clear restrictions on which kinds of behaviour are likely to be associated with each other in parameter space, enabling predictions to be made regarding the possible changes in the oscillation type that may be observed upon changing the model parameters. The analysis suggests that CN is one of a range of oculomotor disorders associated with a pathological saccadic braking signal, and that jerk and pendular nystagmus are the most probable oscillatory instabilities. Additionally, the transition from jerk CN to bidirectional jerk and pendular nystagmus observed experimentally when the gaze angle or attention level is changed is attributed to a gluing bifurcation. This suggests the possibility of manipulating the waveforms of subjects with jerk CN experimentally to produce waveforms with an extended foveation period, thereby improving visual resolution.

Saccadic instabilities and voluntary saccadic behaviour.

Primary gaze fixation is never perfectly stable but can be interrupted by involuntary, conjugate saccadic intrusions (SI). SI have a high prevalence in the normal population and are characterised by a horizontal fast eye movement away from the desired eye position, followed, after a variable duration, by a return saccade or drift. Amplitudes are usually below 1 degrees and they often exhibit a directional bias. The aim of the present study was to investigate the aetiology of SI in relation to saccadic behaviour. It was hypothesised that if SI resulted from deficits in the saccadic system (i.e. reduced inhibitory mechanisms), changes in voluntary saccade behaviour may be apparent and related to SI frequency. To examine this, synchrony (no gap), gap, overlap and antisaccade tasks were conducted on ten normal subjects. No significant correlations were found between SI frequency and voluntary saccade latencies, the percentage of express saccades, or the percentage of antisaccade errors. In addition, no significant correlations were found between SI directional biases and saccade latency directional biases, express saccade biases or antisaccade error biases. These results suggest that an underlying alteration to saccadic behaviour is unlikely to be involved in SI production, and that the SI command signal may arise from the influence of attention on an intact saccadic system. Specifically, descending corticofugal signals relating to attention level and orientation may alter the balance between fixation and saccade generation, so determining SI characteristics.

Characteristics of saccadic intrusions.

Primary fixation is never perfectly stable, but is frequently interrupted by slow drifts, microsaccades and saccadic intrusions (SI). SI are involuntary, conjugate movements which take the form of an initial fast movement away from the desired eye position and followed after a short duration, by either a return secondary saccade or a drift. The purpose of this study was to examine the prevalence and metrics of SI in a population of 50 healthy subjects. Using both one and two dimensional recordings we find that all 50 members of the subject group exhibited SI. The SI were bilateral, conjugate and horizontal. No purely vertical SI were detected when examined in three subjects. SI amplitude mean and range was 0.6 degrees +/-0.5 degrees, 0.1 degrees -4.1 degrees; SI frequency mean and range was 18.0+/-14.3 per min, 1.0-54.8 per min; SI duration mean and range was 225+/-150, 20-870 ms. The mean SI amplitude and frequency when SI<0.5 degrees were removed was 0.97 degrees +/-0.56 degrees and 7.0+/-11.4 per min respectively. Age was positively correlated with SI amplitude (p<0.01), but there was no correlation between age and SI frequency. Three of four types of SI monophasic square wave intrusions (MSWI), biphasic square wave intrusions (BSWI) and double saccadic pulses (DSP) were found to be exclusively saccadic, whilst the fourth type, the single saccadic pulses (SSP), were confirmed to exhibit a slow secondary component. MSWI were the most frequently observed SI occurring in 47 out of 50 (94%) of the subjects with a mean amplitude, frequency and duration of 0.7 degrees +/-0.5 degrees, 11.5+/-11.6 per min, and 255+/-147 ms respectively. Mean amplitudes and frequencies for BSWI (n=20), SSP (n=11) and DSP (n=34) were found to be 0.50 degrees +/-0.2 degrees, 1.2+/-2.5 per min; 0.40 degrees +/-0.20 degrees, 0.4+/-1.0 per min and 0.3 degrees +/-0.4 degrees, 5.0+/-8.7 per min respectively. No differences in MSWI characteristics were found between binocular and monocular viewing. Possible explanations for SI occurrence include experimental viewing conditions, subject fatigue and covert shifts in attention.

Motor and sensory characteristics of infantile nystagmus.

BACKGROUND/AIMS: Past studies have explored some of the associations between particular motor and sensory characteristics and specific categories of non-neurological infantile nystagmus. The purpose of this case study is to extend this body of work significantly by describing the trends and associations found in a database of 224 subjects who have undergone extensive clinical and psychophysical evaluations. METHODS: The records of 224 subjects with infantile nystagmus were examined, where 62% were idiopaths, 28% albinos, and 10% exhibited ocular anomalies. Recorded variables included age, mode of inheritance, birth history, nystagmus presentation, direction of the nystagmus, waveform types, spatial and temporal null zones, head postures and nodding, convergence, foveation, ocular alignment, refractive error, visual acuity, stereoacuity, and oscillopsia. RESULTS: The age distribution of the 224 patients was between 1 month and 71 years, with the mean age and mode being 23 (SD 16) years and 16-20 years respectively. By far the most common pattern of inheritance was found to be autosomal dominant (n = 40), with the nystagmus being observed by the age of 6 months in 87% of the sample (n = 128). 139 (62%) of the 224 subjects were classified as idiopaths, 63 (28%) as albinos, and 22 (10%) exhibited ocular anomalies. Conjugate uniplanar horizontal oscillations were found in 174 (77.7%) of the sample. 32 (14.3%) had a torsional component to their nystagmus. 182 (81.2%) were classed as congenital nystagmus (CN), 32 (14.3%) as manifest latent nystagmus (MLN), and 10 (4.5%) as a CN/MLN hybrid. Neither CN nor MLN waveforms were related to any of the three subject groups (idiopaths, albinos, and ocular anomalies) MLN was found in idiopaths and albinos, but most frequently in the ocular anomaly group. The most common oscillation was a horizontal jerk with extended foveation (n = 49; 27%). The amplitudes and frequencies of the nystagmus ranged between 0.3-15.7 degrees and 0.5-8 Hz, respectively. Periodic alternating nystagmus is commonly found in albinos. Albino subjects did not show a statistically significantly higher nystagmus intensity when compared with the idiopaths (p>0.01). 105 of 143 subjects (73%) had spatial nulls within plus or minus 10 degrees of the primary position although 98 subjects (69%) employed a compensatory head posture. Subjects with spatial null zones at or beyond plus or minus 20 degrees always adopted constant head postures. Head nodding was found in 38 subjects (27% of the sample). Horizontal tropias were very common (133 out of 213; 62.4%) and all but one of the 32 subjects with MLN exhibited a squint. Adult visual acuity is strongly related to the duration and accuracy of the foveation period. Visual acuity and stereoacuity were significantly better (p<0.01) in the idiopaths compared to the albino and ocular anomaly groups. 66 subjects out of a sample of 168 (39%) indicated that they had experienced oscillopsia at some time. CONCLUSIONS: There are strong ocular motor and sensory patterns and associations that can help define an infantile nystagmus. These include the nystagmus being bilateral, conjugate, horizontal uniplanar, and having an accelerating slow phase (that is, CN). Decelerating slow phases (that is, MLN) are frequently associated with strabismus and early form deprivation. Waveform shape (CN or MLN) is not pathognomonic of any of the three subject groups (idiopaths, albinos, or ocular anomalies). There is no one single stand alone ocular motor characteristic that can differentiate a benign form of infantile nystagmus (CN, MLN) from a neurological one. Rather, the clinician must consider a host of clinical features.

Ocular oscillations on eccentric gaze.

Many normal individuals show ocular oscillations on eccentric gaze. This study was designed to investigate the effect of visual disengagement and visual feedback on the nature of these end point oscillations. Three test conditions were examined: target present, target absent and when the target position was determined by the subject's eye position via a variable feedback control system. Feedback gains (i.e. target velocity/eye velocity) ranged from 0, where the target position was decoupled from the subject's eye movements (i.e. the target is stationary on the screen), to +1.0 where the retinal image was stabilised (i.e. the target is driven by the subject's eye movements). Only subjects who exhibited sustained end-point oscillations with no latency were included in the study (n=6). Seven different oscillations including square-wave jerks were recorded in the abducting eye during eccentric gaze of a stationary target. The three most common oscillations were the jerk oscillations, with decelerating, linear or pendular slow phases. A number of additional previously unreported waveforms were also recorded. On removal of the target, the mean drift velocity of the slow phase was greatly reduced. The response to the introduction of a change in the visual feedback was specific to each subject, although in all cases, the end-point oscillations generally were of a lower velocity, and gaze was shifted by up to 8 deg in the direction of the slow phase within the first two seconds. The important role of slow eye movement control for maintaining gaze holding is discussed.

Waveform characteristics of manifest latent nystagmus.

PURPOSE: To examine the waveform characteristics of 37 subjects with manifest latent nystagmus (MLN) and determine the manner in which visual feedback influences the nature of the waveform. METHODS: Binocular recordings of the eye movements of all subjects were undertaken using an infrared tracking system. Subjects viewed the target binocularly and monocularly in primary gaze. The effect of visual feedback on the nature of the MLN waveform was examined by either removing the fixation target or by progressively stabilizing the target in relation to the retina. This progressive stabilization was achieved by feeding back the eye movement signal to move an otherwise stationary target. RESULTS: Four types of MLN were distinguished on the basis of the fixation characteristics seen during binocular and monocular viewing. First, under binocular viewing conditions, subjects could theoretically exhibit stable fixation (type 1 MLN). In addition, three other MLN types were recorded during binocular fixation: conjugate horizontal square-wave jerks (type 2 MLN), conjugate torsional nystagmus (type 3 MLN) and conjugate horizontal jerk MLN waveforms (type 4 MLN). Monocular viewing always gave rise to a conjugate horizontal jerk MLN waveform for each of the four types of MLN. More than 80% of the subjects exhibited either type 3 or type 4 MLN, both of which conform with previous classic descriptions of MLN. Much less common was type 2 MLN. Type 1 MLN (conventionally referred to as a latent nystagmus) appeared to be a rare occurrence. In addition to the two classic linear and decelerating MLN slow phases, four additional slow-phase shapes with either saccadic or pendular elements were recorded and described. Removing visual feedback generally reduced the mean slow-phase velocity and the number of fast phases. For each subject some variability of the slow-phase class was documented from session to session. CONCLUSIONS: Four types of MLN have been described. Their differences are based on their binocular oculomotor behavior, and it is proposed that type 1 MLN and type 4 MLN represent the absolute states and types 2 and 3 the intermediate levels of the MLN spectrum. All types of MLN appear to be strongly visually driven and are largely dependent on the attentional state of the subject and the target conditions. Six different classes of slow phase were found among the four MLN types. The introduction of visual feedback had an immediate effect on the subsequent slow phase or fast phase. It is likely that adaptation mechanisms are in play after a period of visual feedback.

The characteristics of dynamic overshoots in square-wave jerks, and in congenital and manifest latent nystagmus.

Dynamic overshoots are seen after voluntary re-fixation saccades. They are microsaccadic movements which follow primary saccades and have no delay. The purpose of this study was to examine the prevalence and metrics of the dynamic overshoots seen after involuntary saccades. Using infra-red oculography we demonstrate that dynamic overshoots are a common occurrence in physiological square-wave jerks, congenital nystagmus and manifest latent nystagmus and that these overshoots are saccadic in nature and have the same dynamic characteristics as those seen following voluntary saccades. It is therefore likely that they share common neural commands to those dynamic overshoots seen after a volitional saccade. All dynamic overshoots are postulated to be the unwanted consequence of making a saccade and are simulated in a model of fast oculomotor behaviour which is consistent with known experimental results.

Modelling of congenital nystagmus waveforms produced by saccadic system abnormalities.

Models of the mechanisms of normal eye movements are typically described in terms of the block diagrams which are used in control theory. An alternative approach to understanding the mechanisms of normal eye movements involves describing the eye movement behaviour in terms of smooth changes in state variables. The latter approach captures the burst cell firing against motor error (difference between target gaze angle and current gaze angle) phase plane behaviour which is found experimentally and facilitates the modelling of variations in burst cell behaviour. A novel explanation of several types of congenital nystagmus waveforms is given in terms of a saccadic termination abnormality.

Oscillopsia and tolerance to retinal image movement in congenital nystagmus.

PURPOSE: To determine the relationship between retinal image movement (RIM) and oscillopsia in subjects with idiopathic congenital nystagmus (CN). METHODS: Eye movements were recorded using an IRIS infrared system. The eye movement signal was fed back to move an otherwise stationary target on a screen and thereby modify the RIM experienced by each of the five CN subjects. The target was present with either no background (the absolute condition) or a textured background (the relative condition). Feedback gains were varied from -1.0 (i.e., 100% retinal image increase) to +1.0 (i.e., 100% retinal image decrease or complete stabilization), with 0 representing the zero feedback or stationary target condition. In the first experiment, RIM thresholds were determined for a range of feedback values. Using zero feedback, a second experiment measured the detection threshold for absolute and relative motions to a ramp-generated target movement for five CN and five control subjects. RESULTS: Under feedback control spatial constancy broke down for both increased and reduced RIM. The range of spatial constancy was greater for absolute (-0.56 to +0.44) compared with relative (-0.18 to +0.18) RIM. Motion detection thresholds for the CN group were 8 times less sensitive to the absolute and 17 times less sensitive to the relative motion of the target compared with the control group. CONCLUSIONS: These results suggest that in CN subjects perceptual stability is achieved primarily by extraretinal signals.

Gaze orientation during full-field and peripheral field passive optokinesis.

During full-field optokinetic nystagmus the mean position of gaze shifts the eyes in the direction of the fast phase. The driving force for this, pre-supposes that the preferred locus for the position control system, is shifting gaze into the direction where the motion is coming from. In this study, six subjects were examined to determine whether the absence of the central visual field would influence the mean position of gaze during passive optokinesis. Our findings indicate that a full field and a central field restricted to 20 deg, evoked gaze shifts of up to 7 deg into the direction of the fast phase. However, when the central field was masked by either 12.5 deg or 25 deg the mean gaze position was found to be significantly reduced (p < 0.05). This effect was not influenced by the velocity of the stimulus (p > 0.05). These results lead us to conclude that gaze orientation during optokinesis is strongly influenced by the area of retina stimulated. The role of the slow eye movement control system and possible cognitive strategies adopted during selective spatial attention are discussed in the light of this finding.

Monocular optokinetic nystagmus in humans with age-related maculopathy.

AIM: To investigate full field monocular optokinetic nystagmus (OKN) in patients with age-related maculopathy (ARM) and relative central scotoma. METHODS: Six patients aged 59-88 years with bilateral ARM and an aged-matched control group of six patients aged 54-83 years were examined. Visual fields were assessed with a Humphrey field analyser using the threshold 30-1 routine. Monocular full field horizontal optokinetic stimuli were presented on a hemicylindrical screen subtending 172 degrees horizontally and 50 degrees vertically. The stimulus was a projected random dot pattern and three stimulus velocities were used, 30, 50, and 70 degrees/s in both nasalward and temporalward directions. Each trial lasted between 30 and 40 seconds and eye movements were monitored using infrared oculography. RESULTS: The ARM patients had relative central scotomas with an average depth of 10 dB. Neither the ARM nor the age-matched groups displayed any directional preponderance or a buildup of the slow phase eye velocity with time. No statistically significant difference in the gain was found between the two groups (p > 0.05). CONCLUSIONS: Marked central field loss in ARM does not significantly impair OKN gain. This supports the view that complete central retinal integrity is by no means essential and that the peripheral retina provides an important input to the generation of OKN.

Dynamical systems analysis: a new method of analysing congenital nystagmus waveforms.

Congenital nystagmus is an oculomotor disorder in which fixation is disrupted by rhythmical, bilateral involuntary oscillations. Clinically these eye movements have been described with some degree of success in terms of their peak-to-peak amplitude, frequency, mean velocity and waveform shape. However, it has not proved possible to diagnose any underlying pathology from the nystagmus characteristics. Here, we propose a new approach to understanding the nystagmus using dynamical systems theory. Our approach is based on the use of delay embedding techniques, which allow one to relate a time series of scalar observations to the state space dynamics of the underlying dynamical system. Using this approach we quantify the dynamics of the nystagmus in the region of foveation and present evidence to suggest that it is low-dimensional and deterministic. Our results put new constraints on acceptable models of nystagmus and suggest a way to make a closer link between data analysis and model development. This approach raises the hope that techniques originally developed to stabilise chaotic systems, by using small perturbations, may prove useful in the control of nystagmus.

Low contrast letter acuity in age-related maculopathy.

Distance low contrast letter acuity (LCLA) was assessed in six subjects with age-related maculopathy (ARM) and 12 subjects with normal visual function using a series of eight Regan charts ranging in contrast from 4% to 96%. Mean logMAR acuities for letters of 96% contrast were -0.205, -0.053 and 0.748 for the young control group (mean 23.3 years), the age-matched control group (mean 70.3 years) and the ARM subjects (mean 72.3 years), respectively. The LCLA function was found to be significantly better for the six in the young control group compared with the six age-matched controls (P < 0.05). The ARM subjects performed significantly poorer (P < 0.05) at each contrast level down to 20% compared with the age-matched subjects. A constant 0.8 logMAR difference was found between the LCLA function of the age-matched controls and that of the ARM subjects. These results show that, since the low contrast test charts revealed similar differences in visual acuity between the ARM and the age-matched groups, the use of low contrast letter charts do not appear to give any additional information of acuity loss.

Contour interaction in the presence of congenital nystagmus.

Using a computer-generated acuity task, the resolution of vertical and horizontal Landolt Cs was assessed at three levels of contrast, each for three different crowding conditions. Eighteen adults participated; six had congenital idiopathic nystagmus, six were oculocutaneous albinos and six served as controls. Contour interaction was evident when bars were placed 1 gap-width from the Landolt C and was more marked as contrast increased. When scaled to the individual resolution limit, the magnitude of contour interaction displayed by the albinos was not significantly different from the controls, however the idiopaths did exhibit a greater crowding effect than the controls. These results imply that fixation instability is a contributory factor in contour interaction, but only when retinal image velocity is above a certain threshold.

Periodic alternating nystagmus in humans with albinism.

PURPOSE: To quantify the spatial and temporal nature of congenital periodic alternating nystagmus (PAN) and to test the hypothesis that PAN results from a temporal shift in the null zone. METHODS: Twenty-five subjects with oculocutaneous albinism (16 tyrosinase negative and 9 tyrosinase positive) and 7 with ocular albinism (5 x-linked and 2 autosomal recessive) participated in the study. Using infrared oculography, five features of the nystagmus were examined: amplitude, frequency, waveform, beat direction, and temporal nature of the cycle. RESULTS: Twelve subjects (37.5%) exhibited a PAN. The nystagmus waveforms encountered during the PAN active phases were either jerk-with-extended-foveation or pseudocycloid, whereas a variety of oscillations (including triangular and bidirectional) were evident during the quiet phases. For most of the 12 subjects, there was an asymmetric variation in nystagmus intensity during each PAN cycle. None of the 12 demonstrated a convergence null or an abnormal head posture. CONCLUSIONS: PAN is not an uncommon oscillation among humans with albinism. Changes in gaze position markedly influenced the periodicity of the ongoing nystagmus, thus supporting the hypothesis that PAN is the result of a temporal shift in the null zone.